摘要
目的总结下丘脑错构瘤的临床特点和手术治疗体会。方法对我科2013年7月至2018年1月收治的9例诊断为下丘脑错构瘤病例的临床资料进行回顾性分析,并复习相关文献。结果下丘脑错构瘤可引起与病程相关的进展性癫痫性脑病,临床表现为中枢性性早熟、多种癫痫发作及认知和精神方面损害,最具特征性表现为发(痴)笑发作或哭泣样发作但并不是必须存在。脑电图无特异性,详细的核磁共振检查是确诊和分型的重要依据。随着病程的延长,可以在下丘脑错构瘤以外形成新的致痫灶,有时需要电极植入甄别。本组手术病例根据需要采取了不同策略:直接切除下丘脑错构瘤、下丘脑错构瘤加皮层切除、SEEG监测技术下的射频热凝毁损术。经过2个月至4年半的随访,发作均得到较好控制,神经功能损害较术前有不同程度恢复。结论根据典型临床表现,结合详细的影像学检查,诊断不难。根据下丘脑错构瘤的分型结合病史、发作表现采取不同的治疗策略能获得很好的疗效。
Objective To summarize the clinical characteristics and surgical treatment of hypothalamic hamartoma.Methods The clinical data of 9 cases who were diagnosed as hypothalamic hamartoma in our department from July 2013 to January 2018 were retrospectively analyzed and related literature was reviewed.Results Hypothalamic hamartoma can cause progressive epilepsy-related encephalopathy.The clinical manifestations are central precocious puberty,multiple epileptic seizures,and cognitive and mental impairment.The most characteristic manifestation is gelastic seizures or dacrystic seizures,but not every patients has.EEG is not specific and detailed MRI is an important basis for diagnosis and classification.With the prolongation of the course of the disease,new epileptogenic focus can be formed outside of the hypothalamic hamartoma,then electrode implantation is sometimes required to distinguish.In this group of surgical cases,different strategies were taken as required：direct hypothalamic hamartoma resection,hypothalamic hamartoma plus cortical resection,and radiofrequency coagulation and destruction under SEEG monitoring technique.After 2 months to 4 and a half years of follow-up,seizures were well controlled and neurological impairment was restored to varying degrees.Conclusion Based on typical clinical manifestations,it is not difficult to diagnosis hypothalamic hamartoma combining with detailed imaging examination.It can also achieve good results when we take different treatment strategies for different patients according to the classification of the hypothalamic hamartoma and combining with seizure performance.
作者
华刚
谭红平
陈俊喜
张立民
李少春
张伟
郭强
朱丹
Hua Gang;Tan Hongping;Chen Junxi(The Department of Epilepsy Surgery in Guangdong Sanjiu Brain Hospital,Guangzhou,510510)
出处
《立体定向和功能性神经外科杂志》
2018年第2期73-78,共6页
Chinese Journal of Stereotactic and Functional Neurosurgery
关键词
下丘脑错构瘤
发(痴)笑或哭泣样发作
SEEG监测技术
射频热凝毁损术
Hypothalamic hamartoma
Gelastic seizures or Dacrystic seizures
SEEG monitoring technology
Radiofrequency coagulation and destruction
